Clinical, endocrinological and radiological features in a child with McCune-Albright syndrome and pituitary adenoma

被引:0
|
作者
Zumkeller, W
Jassoy, A
Lebek, S
Nagel, M
机构
[1] Univ Halle Wittenberg, Dept Pediat, D-06097 Halle, Germany
[2] Univ Halle Wittenberg, Dept Radiol, D-06097 Halle, Germany
[3] Univ Halle Wittenberg, Dept Orthoped, D-06097 Halle, Germany
[4] Univ Halle Wittenberg, Dept Nucl Med, D-06097 Halle, Germany
关键词
McCune-Albright syndrome; fibrous dysplasia; pituitary adenoma; hyperthyroidism;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
McCune-Albright syndrome is a rare syndrome presenting with polyostotic dysplasia, cafe-au-lait spots and multiple endocrinopathies that is very often combined with precocious puberty. We examined the clinical, endocrinological and radiological features in a boy with McCune-Albright syndrome and pituitary adenoma, X-rays, magnetic resonance (MRT) scan, whole body scintigraphy, single photon emission computer tomography (SPECT) and 3D-reconstruction from bone SPECT was performed to evaluate clinical improvement after treatment with sandostatin and pamidronic acid. After a six-month period of treatment with sandostatin and pamidronate, bone scintigraphy revealed significantly reduced activity, Treatment with bromocriptine and methimazole led to normalization of prolactin and thyroid hormone levels. Mobility of the patient improved. A significant improvement as a result of treatment with sandostatin and pamidronic acid was found in this patient with generalized fibrous dysplasia, So far, this condition has been treated with pamidronate only in adults, but severely affected children also benefit from this treatment regimen.
引用
收藏
页码:553 / 559
页数:7
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